Neurology 14 - CMT Disease + GBS

Question 1

what is Charcot Marie Tooth disease?

Answer 1

not uncommon, auto dominant disease involving peripheral sensory+motor nerve dysfunction

usually presents pre 10 yrs old

Question 2

classic features of Charcot Marie Tooth disease

Answer 2

high foot arches
distal muscle wasting + inverted champagne bottle legs
loss of ankle dorsiflexion
sensory loss peripherally
weakness
loss of tone and reflexes

Question 3

7 causes of peripheral neuropathy

Answer 3

Alcohol
B12 deficiency
Cancer, CKD, CMT
Diabetes, Drugs (isoniazid, amiodarone, cisplatin)
Every vasculitis

Question 4

define Guillain Barre syndrome

Answer 4

acute ascending paralytic neuropathy affecting the peripheral nervous system symmetrically

usually triggered by infection

Question 5

infections associated with GBS

Answer 5

campylobater jejuni, cytomegalovirus, EBV

Question 6

cause of GBS

Answer 6

molecular mimicry between pathogen antigens and antigens on nerve sheaths

(is the theory)

Question 7

presentation of GBS

Answer 7

symmetrical weakness starting in the feet and moving up the body
reduced reflexes
peripheral loss of feeling
neuropathic pain
facial nerve weakness

Question 8

clinical course of GBS

Answer 8

4 weeks post infection
start in feet, progresses for 2 -4 weeks
then lengthy recovery

80% recovery fully

Question 9

diagnosing GBS

Answer 9

Brighton criteria
nerve conduction studies
raised protein in LP

Question 10

management of GBS

Answer 10

IVIG
plasma exchange
supportive care
VTE prophylaxis!! (PE leading cause of death in these patients)